Anti-p200 Pemphigoid

Maike M. Holtsche*, Enno Schmidt, Detlef Zillikens

*Corresponding author for this work

Abstract

Anti-p200 pemphigoid is a rare subepidermal blistering disease first described in 1996. IgG and/or C3 can be detected by direct immunofluorescence microscopy (IF) at the basal membrane zone of the patient’s skin. Circulating antibodies bind to the blister floor of human salt-split skin in indirect IF and, characteristically, recognize a 200 kDa protein by immunoblotting with dermal extract. About 90% of anti-p200 pemphigoid patients have antibodies against laminin γ1 in addition. Clinically, the disease mimics bullous pemphigoid with tense blisters and erosions on the trunk and extremities as well as intense pruritus. In about 40% of patients, mucous membranes are also affected with oral (in 30% of patients), anogenital (in 10%), and conjunctival lesions (in 2%). Cutaneous lesions may heal with hyperpigmentations but without scarring. In some cases, milia are formed. Treatment is based on topical corticosteroids as well as oral prednisolone and immunomodulating drugs like dapsone and doxycycline.

Original languageEnglish
Title of host publicationDiseases of the Oral Mucosa : Study Guide and Review
Number of pages5
PublisherSpringer International Publishing
Publication date01.01.2022
Pages235-239
ISBN (Print)9783030828035
ISBN (Electronic)9783030828042
DOIs
Publication statusPublished - 01.01.2022

Research Areas and Centers

  • Academic Focus: Center for Infection and Inflammation Research (ZIEL)
  • Centers: Center for Research on Inflammation of the Skin (CRIS)

DFG Research Classification Scheme

  • 204-05 Immunology
  • 205-19 Dermatology

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